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Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias

Standard

Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias. / Hoeper, Marius M; Behr, Juergen; Held, Matthias; Grunig, Ekkehard; Vizza, C Dario; Vonk-Noordegraaf, Anton; Lange, Tobias J; Claussen, Martin; Grohé, Christian; Klose, Hans; Olsson, Karen M; Zelniker, Thomas; Neurohr, Claus; Distler, Oliver; Wirtz, Hubert; Opitz, Christian; Huscher, Doerte; Pittrow, David; Gibbs, J Simon R.

in: PLOS ONE, Jahrgang 10, Nr. 12, 02.12.2015, S. Art. e0141911.

Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung

Harvard

Hoeper, MM, Behr, J, Held, M, Grunig, E, Vizza, CD, Vonk-Noordegraaf, A, Lange, TJ, Claussen, M, Grohé, C, Klose, H, Olsson, KM, Zelniker, T, Neurohr, C, Distler, O, Wirtz, H, Opitz, C, Huscher, D, Pittrow, D & Gibbs, JSR 2015, 'Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias', PLOS ONE, Jg. 10, Nr. 12, S. Art. e0141911. https://doi.org/10.1371/journal.pone.0141911

APA

Hoeper, M. M., Behr, J., Held, M., Grunig, E., Vizza, C. D., Vonk-Noordegraaf, A., Lange, T. J., Claussen, M., Grohé, C., Klose, H., Olsson, K. M., Zelniker, T., Neurohr, C., Distler, O., Wirtz, H., Opitz, C., Huscher, D., Pittrow, D., & Gibbs, J. S. R. (2015). Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias. PLOS ONE, 10(12), Art. e0141911. https://doi.org/10.1371/journal.pone.0141911

Vancouver

Hoeper MM, Behr J, Held M, Grunig E, Vizza CD, Vonk-Noordegraaf A et al. Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias. PLOS ONE. 2015 Dez 2;10(12):Art. e0141911. https://doi.org/10.1371/journal.pone.0141911

Bibtex

@article{2e05cc3a71e942d48c7aa8ef2835048c,
title = "Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias",
abstract = "BACKGROUND: Pulmonary hypertension (PH) is a common finding in patients with chronic fibrosing idiopathic interstitial pneumonias (IIP). Little is known about the response to pulmonary vasodilator therapy in this patient population. COMPERA is an international registry that prospectively captures data from patients with various forms of PH receiving pulmonary vasodilator therapies.METHODS: We retrieved data from COMPERA to compare patient characteristics, treatment patterns, response to therapy and survival in newly diagnosed patients with idiopathic pulmonary arterial hypertension (IPAH) and PH associated with IIP (PH-IIP).RESULTS: Compared to patients with IPAH (n = 798), patients with PH-IIP (n = 151) were older and predominantly males. Patients with PH-IIP were treated predominantly with phosphodiesterase-5 inhibitors (88% at entry, 87% after 1 year). From baseline to the first follow-up visit, the median improvement in 6MWD was 30 m in patients with IPAH and 24.5 m in patients with PH-IIP (p = 0.457 for the difference between both groups). Improvements in NYHA functional class were observed in 22.4% and 29.5% of these patients, respectively (p = 0.179 for the difference between both groups). Survival rates were significantly worse in PH-IIP than in IPAH (3-year survival 34.0 versus 68.6%; p<0.001). Total lung capacity, NYHA class IV, and mixed-venous oxygen saturation were independent predictors of survival in patients with PH-IIP.CONCLUSIONS: Patients with PH-IIP have a dismal prognosis. Our results suggest that pulmonary vasodilator therapy may be associated with short-term functional improvement in some of these patients but it is unclear whether this treatment affects survival.TRIAL REGISTRATION: clinicaltrials.gov NCT01347216.",
author = "Hoeper, {Marius M} and Juergen Behr and Matthias Held and Ekkehard Grunig and Vizza, {C Dario} and Anton Vonk-Noordegraaf and Lange, {Tobias J} and Martin Claussen and Christian Groh{\'e} and Hans Klose and Olsson, {Karen M} and Thomas Zelniker and Claus Neurohr and Oliver Distler and Hubert Wirtz and Christian Opitz and Doerte Huscher and David Pittrow and Gibbs, {J Simon R}",
year = "2015",
month = dec,
day = "2",
doi = "10.1371/journal.pone.0141911",
language = "English",
volume = "10",
pages = "Art. e0141911",
journal = "PLOS ONE",
issn = "1932-6203",
publisher = "Public Library of Science",
number = "12",

}

RIS

TY - JOUR

T1 - Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias

AU - Hoeper, Marius M

AU - Behr, Juergen

AU - Held, Matthias

AU - Grunig, Ekkehard

AU - Vizza, C Dario

AU - Vonk-Noordegraaf, Anton

AU - Lange, Tobias J

AU - Claussen, Martin

AU - Grohé, Christian

AU - Klose, Hans

AU - Olsson, Karen M

AU - Zelniker, Thomas

AU - Neurohr, Claus

AU - Distler, Oliver

AU - Wirtz, Hubert

AU - Opitz, Christian

AU - Huscher, Doerte

AU - Pittrow, David

AU - Gibbs, J Simon R

PY - 2015/12/2

Y1 - 2015/12/2

N2 - BACKGROUND: Pulmonary hypertension (PH) is a common finding in patients with chronic fibrosing idiopathic interstitial pneumonias (IIP). Little is known about the response to pulmonary vasodilator therapy in this patient population. COMPERA is an international registry that prospectively captures data from patients with various forms of PH receiving pulmonary vasodilator therapies.METHODS: We retrieved data from COMPERA to compare patient characteristics, treatment patterns, response to therapy and survival in newly diagnosed patients with idiopathic pulmonary arterial hypertension (IPAH) and PH associated with IIP (PH-IIP).RESULTS: Compared to patients with IPAH (n = 798), patients with PH-IIP (n = 151) were older and predominantly males. Patients with PH-IIP were treated predominantly with phosphodiesterase-5 inhibitors (88% at entry, 87% after 1 year). From baseline to the first follow-up visit, the median improvement in 6MWD was 30 m in patients with IPAH and 24.5 m in patients with PH-IIP (p = 0.457 for the difference between both groups). Improvements in NYHA functional class were observed in 22.4% and 29.5% of these patients, respectively (p = 0.179 for the difference between both groups). Survival rates were significantly worse in PH-IIP than in IPAH (3-year survival 34.0 versus 68.6%; p<0.001). Total lung capacity, NYHA class IV, and mixed-venous oxygen saturation were independent predictors of survival in patients with PH-IIP.CONCLUSIONS: Patients with PH-IIP have a dismal prognosis. Our results suggest that pulmonary vasodilator therapy may be associated with short-term functional improvement in some of these patients but it is unclear whether this treatment affects survival.TRIAL REGISTRATION: clinicaltrials.gov NCT01347216.

AB - BACKGROUND: Pulmonary hypertension (PH) is a common finding in patients with chronic fibrosing idiopathic interstitial pneumonias (IIP). Little is known about the response to pulmonary vasodilator therapy in this patient population. COMPERA is an international registry that prospectively captures data from patients with various forms of PH receiving pulmonary vasodilator therapies.METHODS: We retrieved data from COMPERA to compare patient characteristics, treatment patterns, response to therapy and survival in newly diagnosed patients with idiopathic pulmonary arterial hypertension (IPAH) and PH associated with IIP (PH-IIP).RESULTS: Compared to patients with IPAH (n = 798), patients with PH-IIP (n = 151) were older and predominantly males. Patients with PH-IIP were treated predominantly with phosphodiesterase-5 inhibitors (88% at entry, 87% after 1 year). From baseline to the first follow-up visit, the median improvement in 6MWD was 30 m in patients with IPAH and 24.5 m in patients with PH-IIP (p = 0.457 for the difference between both groups). Improvements in NYHA functional class were observed in 22.4% and 29.5% of these patients, respectively (p = 0.179 for the difference between both groups). Survival rates were significantly worse in PH-IIP than in IPAH (3-year survival 34.0 versus 68.6%; p<0.001). Total lung capacity, NYHA class IV, and mixed-venous oxygen saturation were independent predictors of survival in patients with PH-IIP.CONCLUSIONS: Patients with PH-IIP have a dismal prognosis. Our results suggest that pulmonary vasodilator therapy may be associated with short-term functional improvement in some of these patients but it is unclear whether this treatment affects survival.TRIAL REGISTRATION: clinicaltrials.gov NCT01347216.

U2 - 10.1371/journal.pone.0141911

DO - 10.1371/journal.pone.0141911

M3 - SCORING: Journal article

C2 - 26630396

VL - 10

SP - Art. e0141911

JO - PLOS ONE

JF - PLOS ONE

SN - 1932-6203

IS - 12

ER -